title: Effective long-term surgical management of congenital coronary artery fistulas.
abstract: Congenital coronary artery fistula (CAF) is a rare anomaly involving communication between the coronary artery and any cardiac chamber. We retrospectively studied 23 patients with CAF who were surgically treated at 2 institutes over the past 38 years. All patients had continuous murmur and were diagnosed using echocardiography and cardiac catheterization. Eighteen patients had no other heart anomalies, and 5 had an associated anomaly. Fifteen patients were treated using cardiopulmonary bypass via differential surgical approaches (6 patients, CAF orifice closure through cardiac chamber; 6, coronary arteriotomy for CAF orifice closure; and 3, CAF ligation). Eight patients underwent CAF ligation without cardiopulmonary bypass. CAF originated from the right coronary artery in 11 patients; the left coronary artery in 10, and both arteries in 2. Drainage occurred at the following sites: the right ventricle (10 patients), right atrium (6), left ventricle (4), left atrium (2), and pulmonary artery (1). All surgeries were performed through a median sternotomy. There were no mortalities during or after the hospital stay. Aortic root replacement was performed in 1 patient 30 years after the CAF surgery. Three of the 6 patients who underwent coronary arteriotomy for CAF orifice closure showed coronary artery occlusion at the distal coronary arteriotomy site with long-term collateral formation. Surgical management of CAF was thus effective, resulting in 100% long-term survival and closure rates. Dilated CAF-associated vessels have been normalized for the long term. CAF should be therefore considered even in asymptomatic patients because of the risk of future complications.
authors.affiliation: Department of Cardiovascular Surgery, Tokyo Women's Medical University, Japan. tokamura@hotmail.co.jp
authors.forename: Toru
authors.initials: T
authors.lastname: Okamura
authors.forename: Mitsugi
authors.initials: M
authors.lastname: Nagashima
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Yamada
authors.forename: Takeshi
authors.initials: T
authors.lastname: Hiramatsu
authors.forename: Kenji
authors.initials: K
authors.lastname: Yamazaki
mesh_terms: D000293:Adolescent; D000328:Adult; D006328:Cardiac Catheterization; D006348:Cardiac Surgical Procedures / Q000379:methods; D002315:Cardiopulmonary Bypass; D002648:Child; D002675:Child, Preschool; D003331:Coronary Vessels / Q000601:surgery*; D004452:Echocardiography; D005260:Female; D005402:Fistula / Q000151:congenital* / Q000601:surgery*; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000379:methods